Kaiserová Michaela, Menšíková Katerina, Tučková Lucie, Hluštík Petr, Kaňovský Petr
Department of Neurology, University Hospital, Palacky University, Olomouc, Czechia.
Department of Neurology, Faculty of Medicine and Dentistry, Palacky University, Olomouc, Czechia.
Front Neurosci. 2021 Nov 3;15:742042. doi: 10.3389/fnins.2021.742042. eCollection 2021.
Corticobasal syndrome (CBS) is clinically characterized by progressive asymmetric rigidity and apraxia together with symptoms suggestive of cortical involvement and basal ganglia dysfunction. The spectrum of neurodegenerative diseases that can manifest with CBS is wide. The associations of CBS with corticobasal degeneration, progressive supranuclear palsy, Alzheimer's disease, frontotemporal lobar degenerations, Creutzfeldt-Jakob disease, or diffuse Lewy body pathology have been reported. We describe the case of a 71-year-old woman with CBS. The histopathological examination of brain tissue revealed concomitant pathology corresponding to the limbic stage of Lewy-related pathology and the intermediate stage of Alzheimer's-type pathology. To date, there have been only a few cases with a similar combination of pathology manifesting with the CBS phenotype that have been described in the literature. The extent and distribution of pathological changes in these cases were somewhat different from ours, and significance for clinical manifestation was attributed to only one of these pathologies. In our case, we assume that both types of pathology contributed to the development of the disease, considering the presumed specific spread of both types of pathological processes according to Braak's staging. Our case expands the spectrum of neurodegenerative pathological processes that may manifest with the typical CBS phenotype. Also, it points out the importance of identifying specific biomarkers that would enable more accurate differential diagnosis and more accurate determination of the underlying pathological processes of these diseases.
皮质基底节综合征(CBS)的临床特征为进行性不对称性强直和失用症,同时伴有提示皮质受累和基底节功能障碍的症状。可表现为CBS的神经退行性疾病谱很广。已有报道称CBS与皮质基底节变性、进行性核上性麻痹、阿尔茨海默病、额颞叶变性、克雅氏病或弥漫性路易体病理改变有关。我们描述了一例71岁CBS女性患者的病例。脑组织的组织病理学检查显示存在与路易体相关病理改变的边缘期及阿尔茨海默病型病理改变的中期相对应的伴发病理改变。迄今为止,文献中仅描述了少数几例具有类似病理改变组合且表现为CBS表型的病例。这些病例中病理改变的范围和分布与我们的病例略有不同,且其临床表现的意义仅归因于其中一种病理改变。在我们的病例中,考虑到根据Braak分期这两种病理过程可能的特定传播方式,我们推测这两种病理改变均促成了该疾病的发生。我们的病例拓宽了可能表现为典型CBS表型的神经退行性病理过程谱。此外,它还指出了识别特定生物标志物的重要性,这些生物标志物将有助于更准确的鉴别诊断以及更准确地确定这些疾病的潜在病理过程。